Nucci Anita, Burns R Cartland, Armah Tichianaa, Lowery Kristyn, Yaworski Jane Anne, Strohm Sharon, Bond Geoff, Mazariegos George, Squires Robert
Clinical Nutrition Department, Children's Hospital of Pittsburgh, 3705 Fifth Avenue, Pittsburgh, PA 15213, USA.
J Gastrointest Surg. 2008 Mar;12(3):429-35; discussion 435-6. doi: 10.1007/s11605-007-0444-0. Epub 2007 Dec 18.
Management of children with intestinal failure is optimized by interdisciplinary coordination of parenteral and enteral nutrition support, medical management of associated complications, surgical lengthening procedures, and intestinal transplantation. Three hundred eighty-nine pediatric patients have been referred to our center for interdisciplinary assessment of intestinal failure since 1996 (median age=1 year; range 1 day-28.8 years). Factors predictive of weaning from parenteral nutrition without transplantation included increased mean bowel length for patients with gastroschisis (44 vs. 23 cm, p<0.05) and atresia (35 vs. 20 cm, p<0.01) and lower mean total bilirubin for patients with NEC (6.1 vs. 12.7 mg/dL, p<0.05). Others were also more likely to survive if referred with a lower mean total bilirubin (NEC, 7.9 vs. 12.7 mg/dL, p<0.05; pseudo-obstruction, 2.3 vs. 16.3 mg/dL, p<0.01). Patients weaned from parenteral nutrition by 2.5 years after referral achieved 95% survival at 5 years vs. 52% for those not weaned. Bowel lengthening procedures were performed on 25 patients. Eight subsequently weaned from parenteral nutrition without transplantation. Aggressive medical and nutritional intervention along with early referral, intestinal lengthening procedures, and intestinal transplantation in children with intestinal failure dependent on parenteral nutrition can result in the achievement of enteral autonomy and improved survival.
通过肠外和肠内营养支持的跨学科协调、相关并发症的医学管理、手术延长程序和肠道移植，可以优化儿童肠衰竭的管理。自1996年以来，已有389名儿科患者被转诊至我们中心进行肠衰竭的跨学科评估（中位年龄 = 1岁；范围1天至28.8岁）。预测无需移植即可停止肠外营养的因素包括腹裂患者平均肠长度增加（44 vs. 23 cm，p<0.05）和闭锁患者（35 vs. 20 cm，p<0.01），以及坏死性小肠结肠炎患者平均总胆红素较低（6.1 vs. 12.7 mg/dL，p<0.05）。如果转诊时平均总胆红素较低，其他患者也更有可能存活（坏死性小肠结肠炎，7.9 vs. 12.7 mg/dL，p<0.05；假性肠梗阻，2.3 vs. 16.3 mg/dL，p<0.01）。转诊后2.5年内停止肠外营养的患者5年生存率为95%，而未停止的患者为52%。对25名患者进行了肠延长手术。其中8名随后无需移植即停止了肠外营养。对依赖肠外营养的肠衰竭儿童进行积极的医学和营养干预，以及早期转诊、肠延长手术和肠道移植，可以实现肠内自主并提高生存率。
